Hepatoblastoma is an uncommon malignant liver tumour diagnosed usually during the first three years of life. It presents as abdominal mass with elevated alpha fetoprotein levels. The definite diagnosis requires histopathological confirmation. Although conditions like Familial Adenomatous Polyposis (FAP) or Beckwith-Wiedman Syndrome may be associated with hepatoblastomas, storage disorders are uncommonly documented. We describe a rare case of hepatoblastoma with co-existent glycogen storage disease in an infant male who presented with a progressively increasing mass in abdomen along with failure to thrive.